Does referral to a specialist centre improve outcomes in BSTTs?

Introduction

It is estimated that a busy general practitioner will only see one to two patients with sarcoma in their clinical practice lifetime.[1][2][3]. National registries also reveal that a general orthopaedic surgeon can expect to see less than one patient with a primary bone tumour every three years.[4][5] Figures do not exist for the rates of sarcoma patients seen initially by other types of surgeon.

Referral to specialist centres

As it will never be possible to conduct a randomised trial on this topic evidence can only be gained from analysis of series of patients treated within and outside specialist centres. This leads to significant bias in the cases reported as those patients with a good prognosis (for example a patient with a superficial tumour that is readily widely excised) are often not referred whereas those with complex treatment requirements or suspected poorer prognosis are.[6] However, even with these caveats published series consistently report that outcomes are worse when treatment is initiated in non-specialist centres.[5][7][8][9][10][11][12]
Unfortunately, current estimates are that up to half of all patients with soft-tissue sarcoma (STS) are managed outside specialist centres.[1]

As sarcomas are rare, but benign soft tissue tumours are very common, surgery is often undertaken with a plan to perform enucleation or marginal excision. This usually occurs prior to any imaging or biopsy.[13][14][15] These procedures have come to be known as “unplanned surgery”.[16] There is residual tumour found at re-excision in 39-68% of these patients.[17][18][19][20] Re-excision is often a more complex and complicated procedure and the chance for optimal treatment may have been lost by unplanned surgery.[2][21][22][23][24][25] Reported local recurrence rates after unplanned surgery are in the range of 60 to 90%.[16][26][6][27][28]
There is also evidence that disease-specific survival is higher in patients treated in specialist centres.[29][30][2][10][6][18]

Reported series often only consider a few of the possible outcomes of non-referral for specialist treatment. These include the effect of delayed diagnosis, the need for repeated procedures and ultimately the effects on local and distant recurrence.

Evidence summary and recommendations

When biopsies are performed in non-specialist units the errors in diagnosis, non-representative samples and biopsy site complications resulting in alterations in treatment or outcome, have been shown to be between 2–12 times greater than when the biopsy is performed in a specialist centre. Referral of patients to specialist centres results in less unplanned surgery and fewer biopsy-related complications.

Reoperation reveals residual disease in 39-68% of patients referred after their primary excision. Current imaging modalities are unable to reliably predict the presence of residual microscopic disease.

Patients with suspected sarcoma to be referred to a specialist sarcoma unit prior to diagnosis in order to reduce the rates of incomplete excision, reoperation, local recurrence and to improve survival.